Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
40 Cards in this Set
- Front
- Back
|
What is Cystic Fibrosis?
|
A multisytem genetic disease caused by mutations in the Cystic Fibrosis Conductance Regulator (CFTR)
-Primarily affectign the respiratory and gastrointestinal tracts -Respiratory symptoms (cough and SOB) most prevalent |
|
|
Where is the CFTR located?
|
It is a protein that is found in various cells -
Lung epithelium Submucosal glands Pancreas Liver Sweat glands Reproductive tract |
|
|
CTFR Mulations (5)
|
CFTR not synthesized
Defective processing Defective regulation Defective conductance Reduced protein synthesis |
|
|
Signs and symptoms of CF
|
-Very salty-tasting skin
-Persistent coughing, at times with phelgm -Frequent lung infections - Wheezing or SOB -Poor growth/weight gain in spite of a good appetite -Frequent greasy, bulky stools or difficulty in bowel movements |
|
|
DIagnosis or Lab tests
|
-Sweat Chloride Test (standard testing)
-CFTR mutational analysis (DeltaF508) -Nasal Potential Difference (NPD) - correlates with the transport of Na and Cl across cell membranes |
|
|
Pathogenesis of CF
|
1. Deficient defective CFTR
2. Decreased Cl- secretion, increased Na absorption 3. Bronchial obstruction 4. Infection 5. Inflammation |
|
|
Pulmonary disease - What is the predecessor of later infection by P. aeruginosa?
|
Staphlylococcus Aureus
|
|
|
Pulmonary disease - Pseudomonas aeruginosa (most common pathogen)
|
Increased pulmonary inflammation
Negative effect on lung function Mucoid strains - alginate production Biofilm growth Mucoid strains cannot be eradicated |
|
|
Pulmonary disease - Burkholderia Cepacia Complex (2nd most common pathogen)
|
Easily become resistant to ABX during treatment - need three IV drugs to treat (combination therapy)
|
|
|
Pulmonary disease - Stenotrophomonas Maitophilia
|
Chronic infection associated with the development of an immune response against the organism, predicts more exacerbations, but not more progression in decline of lung function
|
|
|
Pulmonary Disease Maintenance - Open the Airway
|
Bronchodilators
-Beta 2 agonists (albuterol) Give prior to treatments to open the airway -Anticholinergics (Ipratropium) |
|
|
Pulmonary Disease Maintenance - Hydrate Secretions
|
7% and 3% Hypertonic Saline for Inhalation
Increase mucocilliary transport SE: excessive cough, transient decrease in FEV1 -Caution in FEV1 < 40% predicted -Bronchospasm - Precede with inhaled beta-agonists |
|
|
Pulmonary Disease Maintenance - Thin Secretions
|
Pulmozyme (rhDNAse/Dornase alpha)
Selectively cleaves DNA released by neutrophils in respiratory tract --> decreased viscoelasticity of lung secretions SE: Voice alterations |
|
|
Pulmonary Disease Maintenance - Mobilize Secretions
|
Airway clearance
-Conventional chest physiotherapy -Active cycle breathing -Forced expiration -Positive expiratory pressure device -High frequency chest oscillation systems |
|
|
Pulmonary Disease Maintenance - Reduce Inflammation
|
Inhaled steroids (Fluticasone) - only for responsive airways
Anti-inflammatory agents (Prednisone 2 mg/kg/day - watch out for cataracts and hyperglycemia) |
|
|
Pulmonary Disease Maintenance - Chronic Suppressive Therapy
|
Inhaled Antibiotics
-TOBI -Aztreonam -Colistin Oral Antibiotics Azithromycin |
|
|
Pulmonary Disease Maintenance - TOBI (Inhaled)
|
Inhaled Tobramycin
300 mg BID on alternate 4 week periods Helps with weight gain For 6 years and older SE: Tinnitus, throat problems and voice problems |
|
|
Pulmonary Disease Maintenance - Aztrenam (Inhaled)
|
Brand Cayston
Indicated for p. aeruginosa 75 mg TID on alternate 4 week periods Precede with bronchodilator For 7 years and older SE: cough, nasal congestion, wheezing Caution: cross reactivity for beta lactam allergy |
|
|
Pulmonary Disease Maintenance - Colistin (Nebulized)
|
Last resort
Alternate/adjunct to TOBI (no clear benefit) 150-300 mg nebulized 2-3 times/day SE: bronchospasm |
|
|
Pulmonary Disease Maintenance - Azithromycin (PO)
|
250 or 500 mg TIW
For 6 years and older Anti-inflammatory action - inhibit alginate production in biofilm SE: N/D, wheezing |
|
|
Pulmonary Disease Maintenance - FEV1
|
Used to measure disease progression
Normal >90% Mildly impaired 70-89% Moderately impaired 40-69% Severely impaired <40% |
|
|
What consititutes an acute exacerbation?
|
There is a long list but these three are the most common:
-Weight loss >1kg -Increased RR or work of breathing or both -Decreased FEV1 of >10% from baseline |
|
|
Pulmonary exacerbation - Treatment of Pseudomonas
|
Pick 2 antipseudomonals:
Ceftazidime, Meropenem, Tiscarcillin/tazobactam, Cipro Duration: 10-14 day duration --> return to baseline |
|
|
Pulmonary exacerbation - Treatment of MSSA/MRSA
|
Pick One:
Vanco, Clindamycin, Nafcillin, Bactrim Duration: 10-14 day duration --> return to baseline |
|
|
How is drug disposition (Vd and Clearance) different in CF pts?
|
-HIgher lean body mass/kg and low adipose tissue will cause increase in Vd of hydophilic drugs
-Increase clearance of beta lactams (may need higher dose) |
|
|
Pulmonary exacerbation - Treatment (general)
|
Tobramycin
Target AUC 80-120 mg*hr/L Once daily administration to reduce nephrotoxicity |
|
|
Pancreatic Insufficiency and Malnutrition in CF
|
-Fecal fat excretion
-Weight loss/poor weight gain -Fat soluble vitamin deficiency -Flatus -Abdominal disconfort |
|
|
Pancreatic Insufficiency and Malnutrition in CF - Pancreatic enzymes
|
Zenpep 5000 IU Lipase
Dosing based on per gram of ingested fat. |
|
|
Pancreatic Insufficiency and Malnutrition in CF - Supplementation
|
ADEK - Fat soluble vitamin supplementation
|
|
|
CF Related DM - Treatment
|
Type 1 and Type 2 DM - these pts crave carbs "Sugar hogs"
Bolus/basal insulin therapy Oral agents are not effective |
|
|
Hepatobilliary Disease in CF - Treatment for Fibrosis, cholestasis and cirrhosis
|
Ursodiol - improves bile flow, displacing toxic bile acids
|
|
|
Hepatobilliary Disease in CF - Treatment of Portal Hypertension
|
Beta blockers
|
|
|
Hepatobilliary Disease in CF - Treatment Liver Failure
|
Transplantation
|
|
|
GI Conditions in CF - Treatment of Distal Intestinal Obstruction Syndrome
|
System rehydration:
-PEG electrolyte solutions (Miralax) -Enemas -Lavage fluid |
|
|
GI Conditions in CF - Treatment of Epigastic Pain, Heartburn and Dyspepsia
|
PPI
H2 Blockers |
|
|
Bone and Joint Disease in CF - Treatment of Low bone mineral density
|
Exercise
Calcium and Vit D Bisphosphonates |
|
|
Bone and Joint Disease in CF - Treatment of Episodic Arthritis
|
NSAIDS (short courses)
|
|
|
Bone and Joint Disease in CF - Treatment of Hypertropic Pulmonary Osteopathy
|
NSAIDS
Analgesics |
|
|
Mental Disorder in CF - Treatment for Depression and Anxiety
|
Psychotherapy
Cognitive behavioral therapy Family therapy Antidepressants |
|
|
New Drug: Ivacaftor (Kalydeco)
|
CFTR potentiator - increases the time that activate CFTR channels at the cell surface remain open, therby increasing chloride and bicarbonate flow.
Only effective in patients with G551D mutation (4.5% of CF patients) |
